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[先天性维生素D抵抗性佝偻病骨组织的病理形态学与矿物质饱和度]

[Pathomorphology and mineral saturation of the bone tissue in congenital vitamin D-resistant rickets].

作者信息

Pavlova M N, Poliakov A N, Berezhnoĭ A P

出版信息

Arkh Patol. 1980;42(7):47-52.

PMID:6251789
Abstract

Fragments of tubular bones obtained in correcting operations from 11 patients aged between 10 and 17 years with congenital vitamin-D-resistant rickets were examined using quantitative microroentgenography, light and electron microscopy. The bone tissue was characterized by reduced mineral saturation of microstructures, marked osteoporosis and numerous resorption foci. The latter were located around osteocytes both in the area of osteones and in inserted plates. Electron microscopy revealed uneven incrustation of the organic matrix with the mineral component as well as disorganization of the ultrastructure of collagen fibrills and associated crystals in the foci of periosteocyte resorption.

摘要

对11例年龄在10至17岁之间的先天性维生素D抵抗性佝偻病患者在矫正手术中获取的管状骨碎片进行了定量微X线摄影、光镜和电镜检查。骨组织的特点是微观结构的矿物质饱和度降低、明显的骨质疏松和大量吸收灶。后者位于骨单位区域和插入板中的骨细胞周围。电镜显示有机基质与矿物质成分的结痂不均匀,以及骨细胞周围吸收灶中胶原纤维和相关晶体的超微结构紊乱。

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1
[Pathomorphology and mineral saturation of the bone tissue in congenital vitamin D-resistant rickets].[先天性维生素D抵抗性佝偻病骨组织的病理形态学与矿物质饱和度]
Arkh Patol. 1980;42(7):47-52.
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Good metabolic control prevents post-surgical imbalance of mineral metabolism in patients with X-linked hypophosphatemic rickets.良好的代谢控制可预防X连锁低磷性佝偻病患者术后矿物质代谢失衡。
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